Congenital intrinsic duodenal obstruction associated with rotational abnormalities of the gut

ABSTRACT

The causes of congenital duodenal obstruction are divided into extrinsic and intrinsic. Intrinsic causes include atresia, stenosis and diaphragm while the commonest extrinsic cause is congenital bands in association with malrotation. The mere presence of malrotation and congenital bands should not be taken for granted that these bands are the cause of obstruction and intrinsic causes should also be excluded. We describe our experience with six children who had rotational abnormalities associated with intrinsic duodenal obstruction. The medical records of 35 children with the diagnosis of intrinsic duodenal obstruction were retrospectively reviewed for age at diagnosis, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment and outcome. The operative findings and procedures were obtained from the operative notes. Six [17.1%] of the 35 children had rotational abnormalities associated with intrinsic duodenal obstruction. All were females. Their age at presentation ranged from 2 days to 1.5 years. Two of them had Down's syndrome and two had congenital heart disease. Two had situs inversus which was diagnosed preoperatively. The remaining 4 had malrotation with congenital bands. The diagnosis in these 4 was made intraoperatively. Intraoperatively, two had duodenal atresia while the other 4 had duodenal diaphragm. In addition to the definitive operative procedure, all had appenedecectomy. Postoperatively, all did well. Rotational abnormalities including situs inversus are among the rare but interesting anomalies associated with intrinsic congenital duodenal obstruction. The presence of malrotation with congenital bands and congenital duodenal obstruction does not exclude the possibility of associated intrinsic cause which should be looked for intraoperatively. This to obviate the possibility of further operative therapy. Appendecectomy should also form part of the initial operative procedure